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At least 1,297 records · Page 72Linked to original sources
PubMed2000

[Fibrocartilaginous dysplasia and tibia vara in young children: presentation of 3 new cases with spontaneous resolution and review of the literature].

Focal fibrocartilaginous dysplasia is a rare and benign condition associated with unilateral tibia vara in toddlers. Three additional cases are reported in children aged 17, 18 and 26 months with spontaneous resolution. The authors discuss, based on a meta-analysis, the natural history of the disorder and review the diagnostic and pathophysiological problems. Since spontaneous remodeling of varus angulation and healing of the bony defect can be expected, biopsy and surgical intervention should be avoided.

Bone Remodeling↗
PubMedDate not supplied

Unilateral exophthalmos associated with severe fibrous dysplasia.

Fibrous dysplasia is a benign developmental anomaly of bone, often affecting craniofacial bones. We report on a 9-year-old boy, who presented for routine checkup. Clinical examination revealed unilateral exophthalmos of his left eye without decrease of visual acuity or double vision. Radiologic studies showed characteristic extensive changes of fibrous dysplasia involving the left frontal bone, left orbital bones, maxillary and sphenoid bones. The patient was started on conservative therapy and the condition of the affected eye remained stable. As fibrous dysplasia of the orbital bones can be a cause of significant dysfunction and a treatable cause of blindness, early diagnosis is very important. These patients are most likely to present with complaints of facial asymmetry, including axial, vertical, or horizontal displacement of the globe, or visual loss. Therefore the ophthalmologist plays an important role in the early diagnosis of fibrous dysplasia.

Antineoplastic Agents↗
PubMed1997

[Focal fibrocartilaginous dysplasia and tibia vara. Apropos of 2 cases. Review of the literature].

Focal cartilaginous dysplasia is a rare condition associated with unilateral tibia vara in young children. The authors report 2 cases. In one patient spontaneous correction occurred at the age of 18 months. In the other case the patient's deformity persisted, and an osteotomy was performed at the age of 36 months. Through these 2 cases and 26 cases of the literature, the authors discuss the healing process and etiology. The most likely exploration is an abnormal development at the insertion of the pes anserinus inducing epiphysiodese like effect. In most cases, the growth plate of the proximal tibial appears to correct the deformity. Surgical correction is necessary after the age of 36 months, if the angular deformation is up to 30 degrees of varus.

Bone Diseases, Developmental↗
PubMed1993

Aggressive fibrous dysplasia of the maxillary sinus.

Fibrous dysplasia is usually a slowly progressive, benign disease that develops over several years and presents with deformity or mild symptomatology. Five of 34 patients (ages 4-21 years), who were subsequently diagnosed histologically as having fibrous dysplasia of the maxillary sinus, rapidly developed soft tissue masses of the malar region over a period of less than 4 months with accompanying pain (2 patients) and nasal obstruction and exophthalmos (2 patients). Each was clinically suspected of having a sarcoma; two had been thought to have an "osteofibrosarcoma" on initial biopsy at outside hospitals. After resection, all lesions developed regrowth. At histopathologic examination, both initial and recurrent masses proved to be typical fibrous dysplasia with spicules of woven bone in cellular, sometimes vascular, fibrous tissue. No malignant degeneration was found. On conventional radiography, aggressive fibrous dysplasia produced opacification and expansion of the maxillary sinus and apparent disruption of its wall with an associated soft tissue mass. Computed tomography (CT) demonstrated voluminous heterogeneous masses with "ground glass appearance", calcifications, areas of enhancement, low attenuation, cystic areas, and a thinned, sometimes interrupted, maxillary wall. Despite the aggressive clinical course for both initial and recurrent lesions, the CT findings of a "ground glass" mass with calcifications surrounded by a maxillary sinus wall, even if incomplete, can suggest the diagnosis of aggressive fibrous dysplasia.

Adolescent↗
PubMed1998

Prenatal ultrasonography: clinical and radiological findings in a boy with fibrochondrogenesis.

Fibrochondrogenesis, a rare lethal chondrodysplasia has been reported on nine patients. We report on a boy with fibrochondrogenesis whose parents were second cousins. Prenatal ultrasonography performed at 22 weeks of gestation revealed an intrauterine growth retardation, an apparently large head, an hypoplasia of the thorax, a prominent abdomen, rhizomelic limbs, and wide metaphysis. The latest have never been reported in other lethal dysplasias.

Bone and Bones↗
PubMed1997

Malignant fibrous histiocytoma degeneration in a patient with facial fibrous dysplasia.

Seventy-two patients with craniofacial fibrous dysplasia were treated during a period of 18 years. Only 1 patient (1.4%) developed malignant degeneration into malignant fibrous histiocytoma. Total excision including the gross mass and adjacent facial bone was performed at that time, however the patient refused an orbital exenteration and postoperative adjuvant therapy. Tumor recurrence developed 2 years later and the patient died. To our knowledge this is the first report describing malignant fibrous histiocytoma degeneration from a fibrous dysplasia localized to the facial bones. Although complete curative treatment could not be undertaken in this case, some conclusions can be drawn regarding the clinical course of this uncommon entity.

Adult↗